What does COVID-19 mean for the Diamond Blackfan Anemia community?

Frequently Asked Questions

 

A few key points:

1 – Coronavirus disease or COVID-19 is caused by a novel coronavirus (meaning that this virus has never infected humans before and there is no immunity in the general population). Coronaviruses are a large family of viruses that are common in people and many different species of animals. Rarely, animal coronaviruses can infect people and then spread among people such as with the Middle Eastern Respiratory Syndrome (MERS-CoV) and the Severe Acute Respiratory Syndrome (SARS-CoV), and now with this new virus, named SARS-CoV-2 that is commonly known as COVID-19; the name used in the following questions/answers. 

2 – The answers provide general knowledge about the novel coronavirus disease called COVID19. The information comes from our growing clinical experience and from reliablesources such as the CDC and NIH and others.

3 – If you have questions about possible symptoms of COVID-19 infection that you or your child are experiencing, please speak to your or your child’s provider.

4 – Reported COVID-19 have ranged from mild symptoms to severe illness and death in the general population.

5 – Symptoms may appear 2-14 days after exposure and usually include fever, cough, shortness of breath and muscle aches. Some patients may be without any symptoms (asymptomatic) and some will have worsening symptoms and require hospitalization.

6 – Do not take any medications on your own to prevent or treat COVID-19. There are no medications that are proven to work to prevent COVID-19 infection (prophylactically) at this time and the medical community is still learning what the best regimen is to treat COVID-19.

7 – Until there are full testing capabilities and a vaccine there may be another wave of infection. For this reason the DBAR would like to gather information on any DBA patient infected with COVID-19 so we can accumulate a list of symptoms, risk factors and outcomes. If you or your child with DBA have been diagnosed with COVID-19, please complete the COVID-19 survey or call/email the DBAR.

 

Topic 1: Questions regarding which patients with DBA are considered “high risk”

Which DBA patients are considered to be at higher risk than the general public with respect to contracting and succumbing to COVID-19?


In the general population, older age, obesity, high blood pressure, diabetes, pre-existing lung disease, compromised immune system and/or cardiac disease have been risk factors for getting very sick with COVID-19. Newer data are indicating that African Americans may also have an increased risk. We do not have any data for DBA patients so this question cannot be answered precisely at this time. With regards to contracting the virus those patients on high-dose steroids and patients with known immune deficiency may be at higher risk. We also theorize that patients with high iron burdens, in particular with cardiac disease from iron overload or with diabetes from iron overload, as well as older patients may be at higher risk of COVID-19 complications.




Is a DBA patient on low dose prednisone with a normal WBC count at greater risk than a person without DBA? Is there a maintenance dose of steroids below which they are no longer considered immunocompromised?


The physicians in DBA community considers the maximal dose of steroids should be less than 0.5 mg/kg/day or 1 mg/kg every other day. This dose is not considered immunosuppressive and, in fact, at these doses, patients can receive live virus vaccines without increased risk.




If a DBA patient is in remission, would he/she still be considered at a higher risk because he/she has an underlying condition?


In general DBA patients are not immune suppressed so most patients may be considered at regular risk. However, this depends on the dose of steroids if the patient is steroid dependent. It also depends on the severity of the iron overload if the patient is transfusion dependent, and the patient’s overall health. This includes whether the patient has endocrinologic problems such as diabetes or decreased thyroid function, or decreased heart function. Each patient has their own individual risk profile that needs to be taken into consideration.




Does being iron overloaded or chelating put a DBA patient at higher risk?


Chelation does not appear to put a patient at any risk for viral infection. However, iron overload can put you at risk for certain bacterial infections and can put you at high risk for endocrine disorders like diabetes, low thyroid hormone, a poor stress response, heart failure, and heart rhythm abnormalities. Individual risk profiles must be taken into consideration.




Many DBA patients never had T-cell or IGG studies done. If a patient hasn’t had those tests, should it be assumed that he/she is immunocompromised just to be safe?


No – DBA is, in general, NOT an immune suppressive disease. Patients with a few RP mutations may have immune suppression. A history of infections should be considered in assessing an individual risk profile. If the patient is on low steroid dose or has low iron burden, then they are not in general significantly immunocompromised.

Some DBA patients may havelow white blood cell (WBC) counts and low neutrophil counts (also called neutropenia). Most patients with DBA do not have severe clinical neutropenia. This means that usually if the patient develops a bacterial infection the WBC and neutrophil count will increase or will functionally be able to fight the bacterial infection. Also having neutropenia does not make a person immunocompromised. Your immune system mostly comes from your lymphocytes. Our body uses lymphocytes to fight viruses so the neutropenia in some DBA patients should not make patients more susceptible to COVID-19.





Topic 2: Taking Precautions against COVID-19

Is the blood supply safe?


Individuals are not at risk of contracting COVID-19 through the blood donation process or via a blood transfusion, since respiratory viruses are generally not known to be transmitted by donation or transfusion. The U.S. Food and Drug Administration continues to report that there have been no reported or suspected cases of transfusion-transmitted COVID-19 to date. In addition, no cases of transfusion-transmission were ever reported for the other two coronaviruses that emerged during the past two decades (MERS and SARS). Also routine blood donor screening measures – which may include travel deferrals – are already in place to prevent individuals with clinical respiratory infections from donating blood and ensuring the safety of the blood supply.




Should we still transfuse at our normal hemoglobin level, or should we try to go longer in between transfusions, because of the possible blood shortage?


The patient with DBA should continue his or her usual transfusion schedule unless the local blood bank reports a specific local shortage. If you or your child becomes ill with COVID-19, it would be better to have a good hemoglobin level.




Do you think the blood supply will dwindle to the point where we will have problems getting blood?


No. There are wonderful people who continue to donate and COVID-19 is not affecting areas nationally at the same time. To date there are no reported shortages.




We have come across doctors who want to limit the need for blood transfusions and suggested the use of steroids. What would you advise as having the lowest risk for DBA patients at this time?


It is best to continue the regimen that the patient is on at present. It is also not the best time to try steroids which are immune suppressive at the higher doses needed.




Should chelation be stopped with any fever? Is it the same recommendation for either desferrioxamine (Desferal®) or deferasirox (Jadenu®/Exjade®)?


Chelation with either desferioxamine or deferasirox is usually held for elevated liver function tests and/or significant increases in kidney function tests but not generally for fever alone.




We usually do a yearly MRI for liver and heart iron measurements. What should we do now?


Due to the risk of COVID-19 exposure it is recommended that routine MRIs are delayed until after the pandemic. Remember chelators only work if they are prescribed and taken at the proper dose and schedule.




I read that it is a good idea to start taking zinc and Vitamin C to help protect against COVID-19. Are these ok to take for the short term while chelating? If they wouldn’t hurt, why not try?


There was a laboratory study back in 2010 that showed that zinc inhibited the activity and replication of the SARS coronavirus. Zinc has also been reported to possibly reduce the duration of the common cold. However, use of zinc lozenges has not been proven effective for COVID-19. Too much zinc is toxic so please be careful if taken.

Vitamin C deficiency is associated with increased susceptibility to infections, a less robust immune response, and poor wound healing. However, there is no evidence that high doses of Vitamin C can prevent or treat COVID-19. In fact, DBA patients who receive chronic transfusions and chelation therapy must be careful not to take extra vitamin C. It can make the iron chelator work more efficiently, but may allow the iron to move around the body. This free-moving iron may deposit in the heart if chelation is not continuous. Therefore vitamin C should only be used in transfusion dependent DBA patients on chelation under very close supervision by a physician. For example, vitamin C can be harmful is taken with chelation that is intermittent like Desferal® given4-5 days per week.




In critical COVID-19 cases, ferritin is apparently elevated. If a DBA patient becomes infected, is there anything admitting doctors need to know about how DBA patients might present regarding ferritin levels in the body and how they are measured?


Ferritin is an acute phase reactant so, in viral and bacterial illnesses, patients will have a rise in ferritin levels. Providers should be aware that in transfused DBA patients with already elevated baseline ferritin levels, the ferritin level may be much higher with a COVID-19 infection. The ferritin level must not be over-interpreted in a transfused DBA patient.





Topic 3: Questions about transfusion dependent DBA patients

Is the blood supply safe?


Individuals are not at risk of contracting COVID-19 through the blood donation process or via a blood transfusion, since respiratory viruses are generally not known to be transmitted by donation or transfusion. The U.S. Food and Drug Administration continues to report that there have been no reported or suspected cases of transfusion-transmitted COVID-19 to date. In addition, no cases of transfusion-transmission were ever reported for the other two coronaviruses that emerged during the past two decades (MERS and SARS). Also routine blood donor screening measures – which may include travel deferrals – are already in place to prevent individuals with clinical respiratory infections from donating blood and ensuring the safety of the blood supply.




Should we still transfuse at our normal hemoglobin level, or should we try to go longer in between transfusions, because of the possible blood shortage?


The patient with DBA should continue his or her usual transfusion schedule unless the local blood bank reports a specific local shortage. If you or your child becomes ill with COVID-19, it would be better to have a good hemoglobin level.




Do you think the blood supply will dwindle to the point where we will have problems getting blood?


No. There are wonderful people who continue to donate and COVID-19 is not affecting areas nationally at the same time. To date there are no reported shortages.




We have come across doctors who want to limit the need for blood transfusions and suggested the use of steroids. What would you advise as having the lowest risk for DBA patients at this time?


It is best to continue the regimen that the patient is on at present. It is also not the best time to try steroids which are immune suppressive at the higher doses needed.




Should chelation be stopped with any fever? Is it the same recommendation for either desferrioxamine (Desferal®) or deferasirox (Jadenu®/Exjade®)?


Chelation with either desferioxamine or deferasirox is usually held for elevated liver function tests and/or significant increases in kidney function tests but not generally for fever alone.




We usually do a yearly MRI for liver and heart iron measurements. What should we do now?


Due to the risk of COVID-19 exposure it is recommended that routine MRIs are delayed until after the pandemic. Remember chelators only work if they are prescribed and taken at the proper dose and schedule.




I read that it is a good idea to start taking zinc and Vitamin C to help protect against COVID-19. Are these ok to take for the short term while chelating? If they wouldn’t hurt, why not try?


There was a laboratory study back in 2010 that showed that zinc inhibited the activity and replication of the SARS coronavirus. Zinc has also been reported to possibly reduce the duration of the common cold. However, use of zinc lozenges has not been proven effective for COVID-19. Too much zinc is toxic so please be careful if taken.

Vitamin C deficiency is associated with increased susceptibility to infections, a less robust immune response, and poor wound healing. However, there is no evidence that high doses of Vitamin C can prevent or treat COVID-19. In fact, DBA patients who receive chronic transfusions and chelation therapy must be careful not to take extra vitamin C. It can make the iron chelator work more efficiently, but may allow the iron to move around the body. This free-moving iron may deposit in the heart if chelation is not continuous. Therefore vitamin C should only be used in transfusion dependent DBA patients on chelation under very close supervision by a physician. For example, vitamin C can be harmful is taken with chelation that is intermittent like Desferal® given4-5 days per week.




In critical COVID-19 cases, ferritin is apparently elevated. If a DBA patient becomes infected, is there anything admitting doctors need to know about how DBA patients might present regarding ferritin levels in the body and how they are measured?


Ferritin is an acute phase reactant so, in viral and bacterial illnesses, patients will have a rise in ferritin levels. Providers should be aware that in transfused DBA patients with already elevated baseline ferritin levels, the ferritin level may be much higher with a COVID-19 infection. The ferritin level must not be over-interpreted in a transfused DBA patient.





Topic 4: Questions about steroid dependent DBA patients

Should we refrain from starting an initial steroid trial during this time (within the next 6 months)? I have heard that steroids can make the immune system weaker.


It may be wise to refrain from starting high dose steroids until the COVID-19 risk decreases in your area. Generally at one year of age, the patient receives the routine live vaccines. About one month later the patient undergoes a bone marrow aspirate and biopsy, if they have not had it done as a baseline before, and then starts a trial with steroids. At this time many institutions are not doing routine procedures so bone marrow exams cannot be done. Also patients who start high dose steroids require ER visits and/or admission for any fever and will need an increase number of visits to the clinic to monitor the side effects of the steroids. Extending the duration of transfusions for a few months until the COVID-19 risk has decreased is not dangerous.




Should we be concerned about the steroid stock?


There is no reported risk of Prednisone or Prednisolone shortage at this time.




I have read that giving steroids can make COVID-19 worse. If my child gets it, should we not do the steroid stress dose protocol like we normally do for illness?


Steroids have been used to treat the lung inflammation with COVID-19 so there should not be fear to use steroids. Stress dose steroids when a steroid dependent patient gets a fever can be vital. In fact adrenal insufficiency should always be treated in the face of a fever as adrenal insufficiency can develop into a critical situation. Both chronic steroid use and iron overload can contribute to adrenal insufficiency.




Are patients with secondary adrenal insufficiency at higher risk of severe disease or complications with COVID-19?


Adrenal insufficiency must be diagnosed and treated immediately in the face of high fevers associated with any infections, including COVID-19. Adrenal insufficiency in and of itself is a risk factor during any stress.





Topic 5: Questions about DBA patients in remission and transplant patients

Is the blood supply safe?


Individuals are not at risk of contracting COVID-19 through the blood donation process or via a blood transfusion, since respiratory viruses are generally not known to be transmitted by donation or transfusion. The U.S. Food and Drug Administration continues to report that there have been no reported or suspected cases of transfusion-transmitted COVID-19 to date. In addition, no cases of transfusion-transmission were ever reported for the other two coronaviruses that emerged during the past two decades (MERS and SARS). Also routine blood donor screening measures – which may include travel deferrals – are already in place to prevent individuals with clinical respiratory infections from donating blood and ensuring the safety of the blood supply.




Should we still transfuse at our normal hemoglobin level, or should we try to go longer in between transfusions, because of the possible blood shortage?


The patient with DBA should continue his or her usual transfusion schedule unless the local blood bank reports a specific local shortage. If you or your child becomes ill with COVID-19, it would be better to have a good hemoglobin level.




Do you think the blood supply will dwindle to the point where we will have problems getting blood?


No. There are wonderful people who continue to donate and COVID-19 is not affecting areas nationally at the same time. To date there are no reported shortages.




We have come across doctors who want to limit the need for blood transfusions and suggested the use of steroids. What would you advise as having the lowest risk for DBA patients at this time?


It is best to continue the regimen that the patient is on at present. It is also not the best time to try steroids which are immune suppressive at the higher doses needed.




Should chelation be stopped with any fever? Is it the same recommendation for either desferrioxamine (Desferal®) or deferasirox (Jadenu®/Exjade®)?


Chelation with either desferioxamine or deferasirox is usually held for elevated liver function tests and/or significant increases in kidney function tests but not generally for fever alone.




We usually do a yearly MRI for liver and heart iron measurements. What should we do now?


Due to the risk of COVID-19 exposure it is recommended that routine MRIs are delayed until after the pandemic. Remember chelators only work if they are prescribed and taken at the proper dose and schedule.




I read that it is a good idea to start taking zinc and Vitamin C to help protect against COVID-19. Are these ok to take for the short term while chelating? If they wouldn’t hurt, why not try?


There was a laboratory study back in 2010 that showed that zinc inhibited the activity and replication of the SARS coronavirus. Zinc has also been reported to possibly reduce the duration of the common cold. However, use of zinc lozenges has not been proven effective for COVID-19. Too much zinc is toxic so please be careful if taken.

Vitamin C deficiency is associated with increased susceptibility to infections, a less robust immune response, and poor wound healing. However, there is no evidence that high doses of Vitamin C can prevent or treat COVID-19. In fact, DBA patients who receive chronic transfusions and chelation therapy must be careful not to take extra vitamin C. It can make the iron chelator work more efficiently, but may allow the iron to move around the body. This free-moving iron may deposit in the heart if chelation is not continuous. Therefore vitamin C should only be used in transfusion dependent DBA patients on chelation under very close supervision by a physician. For example, vitamin C can be harmful is taken with chelation that is intermittent like Desferal® given4-5 days per week.




In critical COVID-19 cases, ferritin is apparently elevated. If a DBA patient becomes infected, is there anything admitting doctors need to know about how DBA patients might present regarding ferritin levels in the body and how they are measured?


Ferritin is an acute phase reactant so, in viral and bacterial illnesses, patients will have a rise in ferritin levels. Providers should be aware that in transfused DBA patients with already elevated baseline ferritin levels, the ferritin level may be much higher with a COVID-19 infection. The ferritin level must not be over-interpreted in a transfused DBA patient.





Topic 6: Questions about COVID-19 treatment/vaccines

Is the blood supply safe?


Individuals are not at risk of contracting COVID-19 through the blood donation process or via a blood transfusion, since respiratory viruses are generally not known to be transmitted by donation or transfusion. The U.S. Food and Drug Administration continues to report that there have been no reported or suspected cases of transfusion-transmitted COVID-19 to date. In addition, no cases of transfusion-transmission were ever reported for the other two coronaviruses that emerged during the past two decades (MERS and SARS). Also routine blood donor screening measures – which may include travel deferrals – are already in place to prevent individuals with clinical respiratory infections from donating blood and ensuring the safety of the blood supply.




Should we still transfuse at our normal hemoglobin level, or should we try to go longer in between transfusions, because of the possible blood shortage?


The patient with DBA should continue his or her usual transfusion schedule unless the local blood bank reports a specific local shortage. If you or your child becomes ill with COVID-19, it would be better to have a good hemoglobin level.




Do you think the blood supply will dwindle to the point where we will have problems getting blood?


No. There are wonderful people who continue to donate and COVID-19 is not affecting areas nationally at the same time. To date there are no reported shortages.




We have come across doctors who want to limit the need for blood transfusions and suggested the use of steroids. What would you advise as having the lowest risk for DBA patients at this time?


It is best to continue the regimen that the patient is on at present. It is also not the best time to try steroids which are immune suppressive at the higher doses needed.




Should chelation be stopped with any fever? Is it the same recommendation for either desferrioxamine (Desferal®) or deferasirox (Jadenu®/Exjade®)?


Chelation with either desferioxamine or deferasirox is usually held for elevated liver function tests and/or significant increases in kidney function tests but not generally for fever alone.




We usually do a yearly MRI for liver and heart iron measurements. What should we do now?


Due to the risk of COVID-19 exposure it is recommended that routine MRIs are delayed until after the pandemic. Remember chelators only work if they are prescribed and taken at the proper dose and schedule.




I read that it is a good idea to start taking zinc and Vitamin C to help protect against COVID-19. Are these ok to take for the short term while chelating? If they wouldn’t hurt, why not try?


There was a laboratory study back in 2010 that showed that zinc inhibited the activity and replication of the SARS coronavirus. Zinc has also been reported to possibly reduce the duration of the common cold. However, use of zinc lozenges has not been proven effective for COVID-19. Too much zinc is toxic so please be careful if taken.

Vitamin C deficiency is associated with increased susceptibility to infections, a less robust immune response, and poor wound healing. However, there is no evidence that high doses of Vitamin C can prevent or treat COVID-19. In fact, DBA patients who receive chronic transfusions and chelation therapy must be careful not to take extra vitamin C. It can make the iron chelator work more efficiently, but may allow the iron to move around the body. This free-moving iron may deposit in the heart if chelation is not continuous. Therefore vitamin C should only be used in transfusion dependent DBA patients on chelation under very close supervision by a physician. For example, vitamin C can be harmful is taken with chelation that is intermittent like Desferal® given4-5 days per week.




In critical COVID-19 cases, ferritin is apparently elevated. If a DBA patient becomes infected, is there anything admitting doctors need to know about how DBA patients might present regarding ferritin levels in the body and how they are measured?


Ferritin is an acute phase reactant so, in viral and bacterial illnesses, patients will have a rise in ferritin levels. Providers should be aware that in transfused DBA patients with already elevated baseline ferritin levels, the ferritin level may be much higher with a COVID-19 infection. The ferritin level must not be over-interpreted in a transfused DBA patient.